Guzy neuroendokrynne trzustki
Leczenie

Guzy neuroendokrynne trzustki (pNET) wymagają wielokierunkowego podejścia terapeutycznego, uwzględniającego typ guza, stopień zaawansowania, lokalizację oraz stan pacjenta. Leczenie chirurgiczne pozostaje podstawą terapii, z resekcją zalecaną dla guzów >2 cm, inwazyjnych lub z zajęciem węzłów chłonnych, natomiast guzy ≤2 cm mogą być monitorowane. Terapie systemowe obejmują analogi somatostatyny (oktreotyd, lanreotyd) skuteczne w kontrolowaniu objawów hormonalnych i wzrostu guza, a także terapię celowaną: ewerolimus (inhibitor mTOR), sunitynib i kabozantynib (inhibitory kinaz tyrozynowych) wykazujące wydłużenie czasu przeżycia wolnego od progresji (od 5,5 do 13,8 miesiąca). Peptydowa terapia radioizotopowa (PRRT) z lutecium Lu-177 dotatate jest skuteczna u pacjentów z ekspresją receptorów somatostatyny, poprawiając kontrolę choroby i jakość życia. Chemioterapia (np. CAPTEM z medianą PFS 22,7 miesiąca) stosowana jest w guzach zaawansowanych, szczególnie słabo zróżnicowanych lub z wysokim indeksem Ki-67.

Leczenie guzów neuroendokrynnych trzustki

Guzy neuroendokrynne trzustki (pNET) stanowią rzadki, ale istotny klinicznie typ nowotworów trzustki, wymagający wielokierunkowego podejścia terapeutycznego. Leczenie tych guzów zależy od wielu czynników, w tym typu guza, jego stopnia zaawansowania, charakterystyki, lokalizacji, stanu ogólnego pacjenta oraz preferencji pacjenta12. Właściwy dobór metody leczenia może prowadzić do całkowitego wyleczenia, kontroli wzrostu guza, zmniejszenia masy guza, wydłużenia przeżycia oraz łagodzenia objawów miejscowych i tych związanych z nadmiernym wydzielaniem hormonów2.

Leczenie chirurgiczne

Leczenie chirurgiczne jest podstawową metodą leczenia guzów neuroendokrynnych trzustki i jedyną, która może prowadzić do całkowitego wyleczenia23. Decyzja o przeprowadzeniu operacji oraz jej zakresie zależy od wielkości guza, jego lokalizacji, obecności przerzutów oraz stanu ogólnego pacjenta1.

Dostępne procedury chirurgiczne obejmują14:

  • Wycięcie guza (enukleacja) – stosowane przy małych, pojedynczych guzach
  • Dystalną pankreatektomię – usunięcie ogona trzustki, stosowane przy guzach zlokalizowanych w ogonie trzustki
  • Pancreatoduodenektomię (operacja Whipple’a) – usunięcie głowy trzustki wraz z częścią dwunastnicy, przewodu żółciowego i żołądka, stosowane przy guzach głowy trzustki
  • Resekcję wątroby – w przypadku przerzutów do wątroby
  • Chirurgiczne zmniejszenie masy guza (debulking) – gdy całkowite usunięcie nie jest możliwe

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Zgodnie z wytycznymi NCCN (National Comprehensive Cancer Network), resekcja chirurgiczna jest zalecana w przypadku nieczynnych hormonalnie guzów neuroendokrynnych trzustki o wielkości >2 cm, guzów inwazyjnych lub guzów z zajęciem węzłów chłonnych27. W przypadku guzów o wielkości ≤2 cm, które są sporadyczne, bezobjawowe i nieczynne hormonalnie, możliwe jest zastosowanie aktywnej obserwacji3.

Badania wykazały, że nawet w przypadku choroby przerzutowej chirurgiczne zmniejszenie masy guza może poprawić jakość życia i wydłużyć przeżycie, jeśli możliwe jest usunięcie przynajmniej 80% masy guza89.

Aktywna obserwacja

W niektórych przypadkach, szczególnie przy małych, nieczynnych hormonalnie guzach neuroendokrynnych trzustki, które rosną powoli, strategią leczenia może być aktywna obserwacja (tzw. „watch and wait” lub nadzór)1011. Podejście to polega na regularnym monitorowaniu guza za pomocą badań obrazowych i laboratoryjnych, bez natychmiastowego wdrażania leczenia10.

Aktywna obserwacja może być odpowiednim wyborem dla12:

  • Pacjentów z nieczynnym hormonalnie guzem o wielkości <1-2 cm
  • Pacjentów starszych z licznymi chorobami współistniejącymi
  • Osób, dla których ryzyko operacji przewyższa potencjalne korzyści

1313

Leczenie hormonalne

Leczenie hormonalne odgrywa istotną rolę w terapii guzów neuroendokrynnych trzustki, szczególnie guzów czynnych hormonalnie oraz w przypadkach, gdy operacja nie jest możliwa11. Głównym celem tej terapii jest kontrola objawów związanych z nadmiernym wydzielaniem hormonów oraz zahamowanie wzrostu guza14.

Analogi somatostatyny (SSA) są podstawowymi lekami stosowanymi w leczeniu hormonalnym guzów neuroendokrynnych trzustki215. Leki te naśladują działanie naturalnego hormonu somatostatyny, hamując wydzielanie hormonów przez komórki guza oraz spowalniając jego wzrost16. Do najczęściej stosowanych analogów somatostatyny należą13:

  • Oktreotyd – stosowany w postaci iniekcji domięśniowych co miesiąc
  • Lanreotyd – podawany w formie iniekcji podskórnych co 4 tygodnie

1617

Analogi somatostatyny wykazują szczególną skuteczność w kontrolowaniu objawów u pacjentów z guzami typu VIPoma i glukagonoma, natomiast mniejszą skuteczność obserwuje się u pacjentów z somatostatinoma lub gastrinoma2. W badaniach PROMID i CLARINET wykazano, że stosowanie analogów somatostatyny poprawia przeżycie u pacjentów z nieoperacyjnymi, dobrze zróżnicowanymi guzami neuroendokrynnymi trzustki17.

W przypadku specyficznych typów guzów stosuje się również inne formy leczenia hormonalnego1718:

  • W przypadku gastrinomainhibitory pompy protonowej w wysokich dawkach, z lub bez analogów somatostatyny
  • W przypadku insulinoma – diazoksyd (50-600 mg dziennie) jako podstawowa terapia; w przypadkach opornych można rozważyć glikokortykosteroidy, werapamil i difenylohydantoinę
  • W przypadku VIPoma – leczenie wspomagające z dożylnym podawaniem płynów i elektrolitów oraz analogi somatostatyny

1518

Terapia celowana

Terapia celowana stanowi ważną opcję w leczeniu zaawansowanych guzów neuroendokrynnych trzustki. W przeciwieństwie do standardowej chemioterapii, leki stosowane w terapii celowanej działają na specyficzne części komórek nowotworowych, co zwiększa skuteczność leczenia i zmniejsza działania niepożądane1920.

Główne leki stosowane w terapii celowanej guzów neuroendokrynnych trzustki to197:

  • Ewerolimus (Afinitor) – inhibitor mTOR, blokuje białko mTOR biorące udział we wzroście i podziałach komórek. Skuteczność ewerolimusu w leczeniu zaawansowanych guzów neuroendokrynnych trzustki została potwierdzona w badaniu RADIANT-3, w którym wykazano znaczące wydłużenie czasu przeżycia wolnego od progresji w porównaniu z placebo1920.
  • Sunitynib (Sutent) – inhibitor wielu kinaz tyrozynowych, blokuje szereg szlaków sygnałowych związanych ze wzrostem guza i tworzeniem nowych naczyń krwionośnych. W badaniu III fazy wykazano, że sunitynib znacząco wydłuża czas przeżycia wolnego od progresji u pacjentów z zaawansowanymi guzami neuroendokrynnymi trzustki (11,4 miesiąca dla sunitynibu wobec 5,5 miesiąca dla placebo)21.
  • Kabozantynib (CABOMETYX) – inhibitor wielu kinaz tyrozynowych (w tym RET, MET i VEGFR2), spowalniający wzrost guza na różne sposoby. Lek ten został niedawno zatwierdzony przez FDA do leczenia dorosłych pacjentów z guzami neuroendokrynnymi trzustki, którzy otrzymali wcześniejsze leczenie, są niekwalifikujący się do operacji i mają dobrze zróżnicowane guzy22. W badaniu III fazy (CABINET) wykazano, że kabozantynib znacząco poprawia przeżycie wolne od progresji u pacjentów z zaawansowanymi guzami neuroendokrynnymi trzustki (13,8 miesiąca wobec 4,4 miesiąca dla placebo)21.
  • Belzutifan – inhibitor HIF, blokujący białko zwane indukowalnym przez hipoksję czynnikiem 2 alfa (HIF-2a), które wpływa na odpowiedź komórki na niskie stężenie tlenu (hipoksję) i na tworzenie nowych naczyń krwionośnych w guzach. Lek ten może być stosowany u osób z chorobą von Hippla-Lindaua (VHL) z zaawansowanym guzem neuroendokrynnym trzustki19.

Terapia celowana jest zwykle stosowana w przypadku guzów zaawansowanych lub nawracających, gdy inne metody leczenia nie są skuteczne16. Warto zauważyć, że w niektórych przypadkach leki te mogą być stosowane w kombinacji z innymi terapiami, np. ewerolimus z analogami somatostatyny, co może dawać lepsze wyniki niż monoterapia15.

Terapia radioizotopowa (PRRT)

Peptydowa terapia radioizotopowa (PRRT – Peptide Receptor Radionuclide Therapy) jest innowacyjną metodą leczenia zaawansowanych guzów neuroendokrynnych trzustki, szczególnie tych, które wykazują ekspresję receptorów somatostatyny23. Metoda ta łączy cząsteczkę, która wiąże się ze specyficznymi receptorami na komórkach guza, z substancją radioaktywną, tworząc tzw. radiopeptyd, który po podaniu dożylnym dostarcza ukierunkowane promieniowanie bezpośrednio do komórek nowotworowych24.

Głównym lekiem stosowanym w PRRT jest lutecium Lu 177 dotatate (Lutathera), który został zatwierdzony do leczenia zaawansowanych guzów neuroendokrynnych1. Leczenie zwykle obejmuje cztery dawki podawane co osiem tygodni23.

PRRT wykazuje szereg zalet w porównaniu z innymi metodami leczenia2125:

  • Precyzyjne ukierunkowanie na komórki nowotworowe przy minimalnym uszkodzeniu zdrowych tkanek
  • Skuteczność u pacjentów z dobrze zróżnicowanymi, nieoperacyjnymi lub przerzutowymi guzami, które nie odpowiadają na standardowe terapie
  • Minimalne działania niepożądane w porównaniu z chemioterapią
  • Zdolność do kurczenia guza u 20-40% pacjentów z guzami neuroendokrynnymi trzustki

2325

Skuteczność PRRT została potwierdzona w badaniu NETTER-1, w którym wykazano, że pacjenci leczeni Lu-177 dotatate plus oktreotyd mieli znacząco dłuższy czas przeżycia wolny od progresji w porównaniu z grupą kontrolną otrzymującą sam oktreotyd w wysokich dawkach25. Mimo że badanie to koncentrowało się głównie na guzach neuroendokrynnych środkowego odcinka przewodu pokarmowego, jego wyniki przyczyniły się do stosowania tej metody w leczeniu innych guzów wykazujących ekspresję receptorów somatostatyny, w tym guzów neuroendokrynnych trzustki25.

PRRT jest najlepiej stosowana u pacjentów, których guzy wykazują silny wychwyt w badaniu PET/CT z Ga-68 DOTATATE, co zapewnia, że terapia będzie specyficznie ukierunkowana na komórki nowotworowe wykazujące ekspresję receptorów somatostatyny25. W 2022 roku Kanadyjska Agencja ds. Leków i Technologii w Ochronie Zdrowia (CADTH) oficjalnie zaleciła finansowanie terapii PRRT opartej na lutecie w leczeniu nieoperacyjnych lub przerzutowych guzów neuroendokrynnych trzustki26.

Chemioterapia

Chemioterapia jest stosowana w leczeniu guzów neuroendokrynnych trzustki, szczególnie w przypadkach zaawansowanych, szybko rosnących guzów lub gdy inne metody leczenia nie są skuteczne127. Leki chemioterapeutyczne działają poprzez zabijanie komórek nowotworowych lub hamowanie ich podziału28.

Główne schematy chemioterapii stosowane w leczeniu guzów neuroendokrynnych trzustki obejmują1329:

  • Kapecytabina + temozolomid (CAPTEM) – schemat ten wykazał znaczącą skuteczność w leczeniu zaawansowanych guzów neuroendokrynnych trzustki. W randomizowanym badaniu fazy II wykazano, że kombinacja kapecytabiny i temozolomidu jest znacznie bardziej skuteczna niż monoterapia temozolomidem, z medianą przeżycia wolnego od progresji 22,7 miesiąca dla CAPTEM w porównaniu z 14,4 miesiąca dla monoterapii temozolomidem25.
  • Streptozocyna – w kombinacji z doksorubicyną lub 5-fluorouracylem
  • Cisplatyna + etopozyd – stosowane głównie w leczeniu słabo zróżnicowanych raków neuroendokrynnych (NEC), które mają szybko postępujący przebieg kliniczny23
  • Inne leki: dakarbazyna, oksaliplatyna

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Efektywność chemioterapii w leczeniu guzów neuroendokrynnych trzustki zależy od stopnia zróżnicowania guza – jest ona bardziej skuteczna w przypadku guzów słabo zróżnicowanych lub w przypadku guzów z wysokim indeksem proliferacyjnym Ki-673031. Należy jednak zauważyć, że dowody na wpływ chemioterapii na ogólne przeżycie są ograniczone lub niejednoznaczne31.

Ze względu na toksyczność systemową, chemioterapia jest zwykle rezerwowana dla pacjentów, u których inne, mniej toksyczne metody leczenia zawiodły, lub w przypadku agresywnych guzów wymagających szybkiej odpowiedzi terapeutycznej32.

Leczenie przerzutów do wątroby

Przerzuty do wątroby są częstym zjawiskiem w przebiegu guzów neuroendokrynnych trzustki i wymagają specjalistycznego podejścia terapeutycznego1. Dostępne opcje leczenia przerzutów do wątroby obejmują23:

Resekcja chirurgiczna wątroby

Chirurgiczne usunięcie przerzutów do wątroby jest możliwe u pacjentów bez rozległego zajęcia obu płatów wątroby, dysfunkcji wątroby, przerzutów pozawątrobowych lub wysokiego stopnia raka neuroendokrynnego2. Resekcja wątroby może obejmować usunięcie części wątroby zajętej przez nowotwór, przy czym pozostała część wątroby przejmuje funkcje usuniętej części1.

W starannie wyselekcjonowanych przypadkach, zwłaszcza u młodych pacjentów z czynnymi hormonalnie guzami neuroendokrynnymi trzustki, które nie odpowiadają na leczenie medyczne, można rozważyć transplantację wątroby313.

Terapie ukierunkowane na wątrobę

W przypadku guzów nieoperacyjnych dostępne są różne metody terapii ukierunkowanej na wątrobę112:

  • Embolizacja tętnicy wątrobowej (HAE) – procedura polegająca na zmniejszeniu przepływu krwi przez główną tętnicę wątrobową za pomocą drobnych cząstek, co prowadzi do spowolnienia lub zatrzymania wzrostu nowotworu. Inne naczynia krwionośne w wątrobie zapewniają wystarczający przepływ krwi dla prawidłowego funkcjonowania pozostałej części wątroby1.
  • Chemoembolizacja przeztętnicza (TACE) – łączy embolizację z dostarczaniem leków chemioterapeutycznych bezpośrednio do guza. Metoda ta spowalnia przepływ krwi i dostarcza leczenie bezpośrednio do komórek nowotworowych w wątrobie1.
  • Radioembolizacja – wykorzystuje radioaktywne kulki (np. itr-90) do dostarczania ukierunkowanego promieniowania do przerzutów w wątrobie. W badaniu Salem i wsp. (2012) wykazano, że radioembolizacja itrem-90 przerzutów do wątroby guzów neuroendokrynnych zapewniała skuteczną kontrolę guza i wydłużała przeżycie u wielu pacjentów25.
Ablacja

Metody ablacyjne wykorzystują różne techniki do niszczenia komórek nowotworowych w wątrobie133:

  • Ablacja prądem o częstotliwości radiowej (RFA) – wykorzystuje fale energii do podgrzewania i niszczenia komórek nowotworowych. Procedura ta jest wykonywana przy użyciu specjalnej sondy z małymi elektrodami, która jest wprowadzana przez skórę do wątroby. Fale energii przechodzą przez sondę i powodują podgrzanie otaczającej tkanki1.
  • Krioablacja – wykorzystuje ekstremalnie niskie temperatury do zamrażania i niszczenia komórek nowotworowych34.
  • Ablacja mikrofalowa – wykorzystuje mikrofale do podgrzewania i niszczenia komórek guza podczas operacji23.

Radioterapia

Radioterapia wykorzystuje wiązki promieniowania o dużej energii do niszczenia komórek nowotworowych1. Energia może pochodzić z promieni rentgenowskich, protonów lub innych źródeł. Podczas radioterapii pacjent leży na stole, podczas gdy maszyna porusza się wokół niego, kierując promieniowanie na precyzyjnie określone punkty ciała1.

W leczeniu guzów neuroendokrynnych trzustki radioterapia odgrywa jednak ograniczoną rolę i jest stosowana głównie w następujących sytuacjach3530:

  • Leczenie objawów takich jak ból, szczególnie gdy nowotwór dał przerzuty do kości
  • Przypadki, gdy operacja nie jest możliwa lub gdy po operacji pozostał guz resztkowy
  • Jako element terapii skojarzonej z innymi metodami leczenia

Należy zauważyć, że skuteczność radioterapii w guzach neuroendokrynnych trzustki jest często mniej wyraźna niż w innych typach nowotworów36.

Leczenie wspomagające

Leczenie wspomagające (podporowe) ma na celu łagodzenie problemów spowodowanych przez chorobę lub jej leczenie28. W przypadku guzów neuroendokrynnych trzustki leczenie wspomagające może obejmować3434:

  • Leki na wrzody żołądka
  • Leczenie lub leki dostępne bez recepty na biegunkę
  • Płyny dożylne w celu zapobiegania odwodnieniu
  • Leki lub inne strategie utrzymujące poziom cukru we krwi w zdrowym zakresie

W przypadku przełomu rakowiaka, który jest stanem nagłym wymagającym natychmiastowej interwencji, leczenie obejmuje infuzję oktreotydu wraz z antagonistami serotoniny, takimi jak ondansetron, cyproheptadyna i ranitydyna18.

Ponadto, w przypadku pacjentów z niewydolnością zewnątrzwydzielniczą trzustki (PEI), która może wystąpić po resekcji trzustki, podstawowym leczeniem jest enzymatyczna terapia zastępcza trzustki (PERT), która znacznie poprawia przeżycie i jakość życia37.

Nowe kierunki i badania kliniczne

Krajobraz leczenia guzów neuroendokrynnych trzustki stale się rozwija, a badacze poszukują nowych, bardziej skutecznych metod terapeutycznych38. Aktualnie prowadzone są badania w następujących obszarach3935:

Immunoterapia

Immunoterapia wykorzystuje naturalny system odpornościowy organizmu do rozpoznawania i niszczenia komórek nowotworowych40. W przypadku guzów neuroendokrynnych trzustki rola immunoterapii wciąż się rozwija i jest aktywnie badana21.

W przeciwieństwie do niektórych agresywnych nowotworów, które dobrze reagują na leczenie immunologiczne, guzy neuroendokrynne trzustki są zwykle wolno rosnące i nie wywołują silnych odpowiedzi immunologicznych. To sprawia, że są one mniej podatne na standardowe immunoterapie, takie jak inhibitory punktów kontrolnych21. Leki takie jak niwolumab (anty-PD-1) i pembrolizumab działają poprzez blokowanie białek, które komórki nowotworowe wykorzystują do ukrywania się przed układem odpornościowym21.

W badaniu klinicznym testującym pembrolizumab u pacjentów z guzami neuroendokrynnymi trzustki wykazano, że lek jest dobrze tolerowany, ale odpowiedź na leczenie wystąpiła tylko u 6% pacjentów. Co istotne, pacjenci, którzy osiągnęli odpowiedź, mieli zwykle długotrwałą kontrolę choroby, ze wszystkimi odpowiedziami trwającymi co najmniej sześć miesięcy41.

Badacze obecnie eksplorują strategie kombinacyjne w celu poprawy wyników leczenia. Obejmuje to stosowanie immunoterapii razem z innymi metodami leczenia, takimi jak terapia radioizotopowa (PRRT) lub terapie celowane21. Na przykład uszkodzenie spowodowane przez PRRT może eksponować więcej antygenów nowotworowych, czyniąc immunoterapię bardziej skuteczną21.

W fazie wczesnych badań są również spersonalizowane szczepionki mRNA zaprojektowane w celu wywołania odpowiedzi immunologicznej przeciwko specyficznym antygenom nowotworowym u pacjentów z rakiem trzustki. Wstępne wyniki wykazały, że niektórzy pacjenci pozostawali wolni od nowotworu przez dłuższy czas po leczeniu25.

Kombinacje terapeutyczne

Badania nad nowymi kombinacjami leków stanowią obiecujący kierunek w leczeniu guzów neuroendokrynnych trzustki23. Przykłady badanych kombinacji obejmują15:

  • Temsirolimus (inhibitor mTOR) z bewacizumabem (inhibitor VEGF)
  • PRRT w połączeniu z operacją
  • Surufatinib (terapia celowana) z tislelizumabem (immunoterapia) dla guzów neuroendokrynnych trzustki, płuc i przewodu pokarmowego25

Terapie ukierunkowane na markery genetyczne

Zrozumienie genetycznych podstaw różnych podtypów guzów neuroendokrynnych trzustki otwiera nowe możliwości dla terapii dostosowanych do indywidualnych potrzeb15. Przykładowo, ponieważ około 90% nowotworów trzustki ma mutacje KRAS, badane są terapie celowane, takie jak daraxonrasib25.

Badacze w Memorial Sloan Kettering opracowują również nowe podejścia terapeutyczne z wykorzystaniem modelu mysiego, który wykazuje guzy neuroendokrynne trzustki. Model ten będzie wykorzystywany do wczesnych testów nowych terapii lekowych i ukierunkowanych terapii opartych na przeciwciałach35.

Wielodyscyplinarne podejście do leczenia

Leczenie guzów neuroendokrynnych trzustki wymaga wielodyscyplinarnego podejścia, z udziałem specjalistów z różnych dziedzin10. Zespół wielodyscyplinarny (MDT) może obejmować42:

  • Endokrynologów
  • Chirurgów endokrynologicznych
  • Onkologów
  • Radiologów
  • Specjalistów medycyny nuklearnej
  • Patomorfologów
  • Pracowników socjalnych

Taki zespół współpracuje, aby zapewnić kompleksową opiekę uwzględniającą wszystkie potrzeby pacjenta42. Pacjenci leczeni w wyspecjalizowanych ośrodkach o dużej liczbie przypadków wydają się mieć lepsze wyniki niż przeciętni pacjenci42.

Indywidualizacja planu leczenia jest kluczowa i powinna uwzględniać43:

  • Rodzaj guza neuroendokrynnego trzustki (czynny lub nieczynny hormonalnie)
  • Masę guza i jego rozkład
  • Indeks Ki-67 i tempo choroby
  • Dostępne metody leczenia
  • Preferencje pacjenta

Optymalny schemat leczenia systemowego zaawansowanych guzów neuroendokrynnych trzustki zależy głównie od masy guza, indeksu Ki-67 i tempa choroby43. W przypadkach, gdy operacja lub terapia ukierunkowana na wątrobę nie są możliwe, leczenie opiera się na wytycznych ENETS (European Neuroendocrine Tumor Society) i ESMO (European Society for Medical Oncology)43.

Podsumowanie i perspektywy

Leczenie guzów neuroendokrynnych trzustki znacznie ewoluowało w ostatnich latach, oferując pacjentom bardziej ukierunkowane i skuteczne metody terapeutyczne15. Od zabiegów chirurgicznych po terapie celowane i radioizotopowe, dostępne opcje leczenia pozwalają na indywidualne podejście do każdego pacjenta18.

Mimo że guzy neuroendokrynne trzustki stanowią wyzwanie diagnostyczne i terapeutyczne, postęp w zrozumieniu biologii molekularnej tych guzów oraz rozwój nowych metod leczenia dają nadzieję na lepsze wyniki leczenia w przyszłości44. Badania kliniczne nad nowymi terapiami, w tym immunoterapią i kombinacjami lekowych, mogą prowadzić do dalszych postępów w leczeniu tych rzadkich nowotworów5.

Ważne jest, aby pacjenci z guzami neuroendokrynnymi trzustki byli leczeni w wyspecjalizowanych ośrodkach, gdzie mogą uzyskać dostęp do najnowszych metod leczenia i multidyscyplinarnej opieki45. Pacjenci powinni również rozważyć udział w badaniach klinicznych, które mogą oferować dostęp do innowacyjnych terapii22.

Choć całkowite wyleczenie może nie zawsze być możliwe, szczególnie w przypadku zaawansowanej choroby, celem terapii jest kontrola wzrostu guza, zmniejszenie objawów i poprawa jakości życia5. Dzięki postępom w leczeniu, pacjenci z guzami neuroendokrynnymi trzustki mogą żyć dłużej i z lepszą jakością życia niż kiedykolwiek wcześniej5.

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Pancreatic neuroendocrine tumors | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/pancreatic-neuroendocrine-tumors
    Treatment for a pancreatic neuroendocrine tumor depends on the types of cells involved in your cancer, the extent and characteristics of your cancer, your preferences, and your overall health. […] Options may include: […] Surgery. If the pancreatic neuroendocrine tumor is only in the pancreas, treatment usually includes surgery. For cancers in the tail of the pancreas, surgery may involve removing the tail of the pancreas, called a distal pancreatectomy. This surgery leaves the head of the pancreas intact. […] Cancers that affect the head of the pancreas may require the Whipple procedure, also called pancreaticoduodenectomy. This surgery involves removing the cancer and part or most of the pancreas. […] If the cancer spreads to other parts of the body, surgery might be an option to remove it from those locations.
  • #1 Pancreatic neuroendocrine tumors | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/pancreatic-neuroendocrine-tumors
    Peptide receptor radionuclide therapy, also called PRRT. PRRT combines a medicine that targets cancer cells with a small amount of a radioactive substance that’s injected into a vein. The medicine sticks to the pancreatic neuroendocrine tumor cells wherever they are in the body. Over days to weeks, the medicine delivers radiation directly to the cancer cells, causing them to die. […] One PRRT, lutetium Lu 177 dotatate (Lutathera), is used to treat advanced cancers. […] Targeted therapy. Targeted therapy uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. Targeted therapy is used to treat certain advanced or recurrent pancreatic neuroendocrine tumors. […] Radiofrequency ablation. Radiofrequency ablation involves applying energy waves to cancer cells using a special probe with tiny electrodes. Radiofrequency ablation causes the cancer cells to heat up and die. The probe may be inserted directly in the skin or through a cut in the abdomen.
  • #1 Pancreatic neuroendocrine tumors | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/pancreatic-neuroendocrine-tumors
    Radiation therapy. Radiation therapy uses powerful energy beams to kill cancer cells. The energy comes from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body. […] Chemotherapy. Chemotherapy uses strong medicines to kill cancer cells. It’s used in certain situations to treat pancreatic neuroendocrine tumors. […] Pancreatic neuroendocrine tumors most often spread to the liver. Several treatments exist for this, including: […] Removing part of the liver. A surgeon might remove the part of the liver that has cancer. The rest of the liver can take over for the part that’s removed. In certain situations, it might be possible to remove the entire liver and replace it with a liver from a donor. This procedure is called a liver transplant.
  • #1 Pancreatic neuroendocrine tumors | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/pancreatic-neuroendocrine-tumors
    Slowing blood flow to the liver. Slowing blood flow through the liver’s main artery, called the hepatic artery, can slow or stop cancer growth. Other blood vessels in the liver provide enough blood for the rest of the liver to keep working. Often, chemotherapy medicines or radioactive beads are used to block the artery. These methods slow the blood flow and deliver treatment directly to the cancer cells in the liver. […] Destroying cancer cells with heat. Radiofrequency ablation is a procedure that uses energy waves to heat up cancer cells and cause them to die. It’s done using a special probe with tiny electrodes that’s inserted through the skin and into the liver. Energy waves pass through the probe and cause the tissue around it to heat up.
  • #2 Treatment Options for Pancreatic Neuroendocrine Tumors
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6628351/
    The management of pancreatic neuroendocrine tumors (PanNETs) involves classification into non-functional or functional PanNET, and as localized or metastatic PanNET. […] Accurate staging of the disease is essential for determining the appropriate approach to therapy. As cure is only potentially possible with surgical resection of the tumor, it is recommended to remove all localized and limited metastatic disease. However, many patients present with metastatic and/or advanced local disease. In such instances, the goal of therapy is to control tumor growth and/or decrease tumor burden, lengthen survival, and palliate local symptoms and those of hormone excess. This typically requires a multimodal approach, including surgery, liver-directed treatment, and systemic medical therapy. […] Surgery is the only treatment approach that can cure a PanNET. In addition, surgical resection may be used to alleviate symptoms secondary to local mass effect or excess hormone levels.
  • #2 Treatment Options for Pancreatic Neuroendocrine Tumors
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6628351/
    When surgery is indicated, a decision regarding the extent of surgery must be made. A complete surgical resection includes removing the primary tumor and any lymph node metastases. […] The NCCN guidelines recommend in cases of non-functional PanNETs 2 cm, invasive, or node-positive tumors, to complete pancreatoduodenectomy and regional nodes for tumors situated in the head of the pancreas, or distal pancreatectomy with splenectomy and regional nodes, for tumors in the distal pancreas. […] Medical treatment is required in many patients with advanced, recurrent, or metastatic PanNETs that are not candidates for surgical intervention. […] The first consideration in patients with unresectable, functional PanNET, is hormonal control, as hormone overproduction may cause significant morbidity and mortality.
  • #2 Treatment Options for Pancreatic Neuroendocrine Tumors
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6628351/
    There are three main different groups of medical therapies available for control of tumor growth: Somatostatin analogues (octreotide, lanreotide), molecular targeted treatment (everolimus, sunitinib, others), and chemotherapy (capecitabine, 5-fluorouracil, capecitabine, dacarbazine, oxaliplatin, streptozotocin, and temozolomide). […] Somatostatin analogues may be effective in both controlling the symptoms of functional PanNETs, especially VIPomas and glucagonomas, with a lesser effect in patients with somatostatinomas or gastrinomas. […] Two agents have been shown to improve progression-free survival in patients with advanced PanNETeverolimus and sunitnib. […] The common adverse effect of hyperglycemia associated with everolimus may be of particular value in patients with functioning insulinoma and refractory hypoglycemia.
  • #2 Treatment Options for Pancreatic Neuroendocrine Tumors
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6628351/
    The combination of temisirolimus, another mTOR inhibitor, with bevacizumab, a VEGF inhibitor, was studied in a multi-center phase II study of patients with locally advanced or metastatic, well-, or moderately differentiated PanNETs, with evidence of progressive disease. […] In patients with hepatic-dominant disease, liver-directed therapies may be considered. […] Surgical resection of liver metastases is an option for patients without diffuse bilobar involvement, liver dysfunction, extrahepatic metastases, or high-grade neuroendocrine carcinoma. […] In many patients with advanced or metastatic disease, medical treatment is required. This additional treatment may be directed to control the symptoms and/or tumor growth. […] For patients with neuroendocrine carcinoma (NEC), which is poorly differentiated and frequently has a rapidly progressive clinical course, combination chemotherapy of cisplatin and etoposide, or its analogs, is recommended.
  • #3 Treatment Options for Pancreatic Neuroendocrine Tumors
    https://www.mdpi.com/2072-6694/11/6/828
    Surgery is the only treatment approach that can cure a PanNET. In addition, surgical resection may be used to alleviate symptoms secondary to local mass effect or excess hormone levels. […] When surgery is indicated, a decision regarding the extent of surgery must be made. A complete surgical resection includes removing the primary tumor and any lymph node metastases. […] Several studies investigated the outcomes of non-operative management and reported that for non-functioning PanNETs, which are sporadic, asymptomatic, and ≤2 cm, a conservative approach with active surveillance is safe, as in most cases, there was no significant change in the size of the tumors during follow-up. […] The NCCN guidelines recommend in cases of non-functional PanNETs >2 cm, invasive, or node-positive tumors, to complete pancreatoduodenectomy and regional nodes for tumors situated in the head of the pancreas, or distal pancreatectomy with splenectomy and regional nodes, for tumors in the distal pancreas.
  • #3 Treatment Options for Pancreatic Neuroendocrine Tumors
    https://www.mdpi.com/2072-6694/11/6/828
    In patients with hepatic-dominant disease, liver-directed therapies may be considered. […] Surgical resection of liver metastases is an option for patients without diffuse bilobar involvement, liver dysfunction, extrahepatic metastases, or high-grade neuroendocrine carcinoma. […] In highly selected patients, usually young patients with functional PanNET with no response to medical treatment, liver transplantation can be considered. […] Surgery is the only treatment that can cure functional or non-functional PanNETs and in many cases this will be the first modality applied in these patients. […] For patients with non-functioning, small, sporadic, and asymptomatic PanNETs mainly in the head of the pancreas, a conservative approach with active surveillance is feasible, though this is controversial. […] For patients with neuroendocrine carcinoma (NEC), which is poorly differentiated and frequently has a rapidly progressive clinical course, combination chemotherapy of cisplatin and etoposide, or its analogs, is recommended.
  • #4
    http://www.bccancer.bc.ca/books/neuroendocrine-tumors/pancreatic-neuroendocrine-tumors
    Surgical treatment of pancreatic cancer should be undertaken by surgeons having expertise and experience with these tumours. For resectable pNETs: definitive resection of the primary tumour should always be performed whenever technically feasible. For localized, locoregional, or resectable metastatic pNETs, curative surgery should be considered if technically and clinically feasible. For unresectable metastatic pNETs, cytoreductive surgery to achieve maximal debulking and palliation of symptoms should be considered if technically feasible and clinically appropriate. […] Treatment options are based on current evidence. Surgery is the only curative treatment. Indications for surgery depend upon the size and location of the pNET. Surgical procedures include: pancreaticoduodenectomy (Whipples resection: classic or pylorus-preserving), distal pancreatectomy +/- splenectomy, tumour enucleation, enucleation combined with resection, regional lymph node dissection required for 'malignant’ (high grade) pNETs.
  • #5 Treating Pancreatic Neuroendocrine Tumors – Let’s Win Pancreatic Cancer
    https://letswinpc.org/treatments/living-longer-pancreatic-neuroendocrine-tumors/
    They are words you dont often hear together: Metastatic cancer and hope. […] But thats exactly what Dr. Pamela Joy Hodul offers her pancreatic neuroendocrine tumor (PNET) patients; hope, if not for a cure, then for prolonged survival and a good quality of life. Even in the metastatic setting, there are many treatment options. […] Based on information provided by these tests, doctors are presented with several treatment options, Hodul explains. The surgical principles for PNETs are essentially the same as for adenocarcinomas. Tumors contained within the pancreas and not tied up in any blood vessels may be candidates for surgical removal. Larger tumors, or those in difficult locations, might be able to be shrunk with chemotherapy and operated on later. […] Doctors also have additional tools in their kits for neuroendocrine tumors, however. These include injections of medicines that block the hormones feeding the tumors, or of radioactive molecules that bind to peptide receptors and deliver super-targeted therapy.
  • #5 Treating Pancreatic Neuroendocrine Tumors – Let’s Win Pancreatic Cancer
    https://letswinpc.org/treatments/living-longer-pancreatic-neuroendocrine-tumors/
    For those whose tumors have spread to other organs, such as the liver, interventional radiology procedures such as embolization to cut off the tumors blood supply, or ablation to burn it away, may be options. Neuroendocrine cancers often respond well to these interventions, and as long as doctors can continue to debulk the tumors, the cancer can stay at bay for years, adds Hodul. […] The scope of options and varied aspects of the disease in all its personal permutations means specialist advice should be sought, however, especially when developing an initial treatment plan. […] PNET patients should be optimistic as they face the disease, Hodul says. The disease progression is very different from other types of cancer, and should give hope to PNET patients. Treatment can be very beneficial in most cases, even in metastatic disease.
  • #6 Pancreatic Neuroendocrine Tumors: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/21970-pancreatic-neuroendocrine-tumors
    Pancreatic neuroendocrine tumors develop in the endocrine cells in your pancreas. […] Treatment is surgery to remove the tumors or receptor-targeted therapies and other nonsurgical treatments. […] Treatment for early-stage pancreatic NET is a pancreatectomy to remove the tumor. This is a treatment for cancerous and noncancerous tumors. Specific surgeries vary depending on the tumors location in your pancreas. […] Surgery may not be an option if you have an advanced form of pancreatic NET. Your oncologist may recommend other treatments, including: hormone therapy, peptide receptor radionuclide therapy (PRRT), tyrosine kinase inhibitors, chemotherapy. […] Your recovery time will depend on the type of surgery you have. For example, it may take several weeks for you to recover from a Whipple procedure.
  • #7 Treatment Options for Pancreatic Neuroendocrine Tumors – Endocrinology Advisor
    https://www.endocrinologyadvisor.com/features/treatment-options-for-pancreatic-neuroendocrine-tumors/
    In the management of PNETs it is important to differentiate between functional and nonfunctional PNETs in order to determine the course of treatment. […] While treatment is not required in all cases of PNET, surgery is frequently the first-line treatment when there is an indication for intervention. However, when patients with PNETs present with metastatic or locally advanced disease, additional treatment options may include systemic medical therapy, liver-directed treatment, and radiation. […] In cases of resectable disease, surgical resection of the tumor and regional lymph nodes is the only treatment approach that can lead to a cure in patients with PNET. […] The National Comprehensive Cancer Network guidelines recommend surgical intervention for nonfunctional PNETs that are 2 cm, invasive, or node-positive tumors.
  • #7 Treatment Options for Pancreatic Neuroendocrine Tumors – Endocrinology Advisor
    https://www.endocrinologyadvisor.com/features/treatment-options-for-pancreatic-neuroendocrine-tumors/
    When surgical intervention is not expected to accomplish removal of the entire tumor, tumor debulking can be considered. […] Medical treatment may be required in cases of advanced or metastatic disease, with 2 main goals: symptom control and control of tumor growth. […] Various treatment options are available for alleviating symptoms related to functional PNETs in accordance with the specific hormone excess. […] Octreotide and lanreotide are somatostatin analogs that bind mainly to somatostatin receptor type 2 and inhibit hormone secretion from tumor cells. […] Everolimus (Afinitor), an oral mammalian target of rapamycin (mTOR) inhibitor, is a common second-line treatment for patients with progressive metastatic PNETs. […] Sunitinb (Sutent), an oral multitargeted tyrosine kinase inhibitor, may improve overall and progression-free survival and was approved for the treatment of progressive well-differentiated PNETs in patients with unresectable, locally advanced, or metastatic disease.
  • #8 Neuroendocrine Pancreatic Tumor Treatment Options | University of Utah Health
    https://healthcare.utah.edu/the-scope/health-library/all/2016/02/neuroendocrine-pancreatic-tumor-treatment-options
    Neuroendocrine pancreatic tumors are dangerous because they might be potentially cancerous but can go relatively unnoticed for a long period of time. […] Pancreatic neuroendocrine tumors are a rare tumor, like you stated, representing approximately 5% of all tumors of the pancreas. […] So to point out to the audience, even though they’re benign they still produce hormones like insulin or glucagon and that causes abnormal symptoms in the patient and then has to be treated. So generally, even though they’re benign, if they’re functional they are taken out or treated. […] There are multiple treatment options, obviously surgical resection being one of the first and foremost. […] Our division gets involved when these lesions are metastatic to the liver. […] There are excellent results here at The U in regards to neuroendocrine tumor resection, in regards to debulking of these tumors, and it has been well-described that these tumors, if debulked and surgically managed, can be beneficial to the patient long-term.
  • #9 Neuroendocrine Pancreatic Tumor Treatment Options | University of Utah Health
    https://healthcare.utah.edu/the-scope/health-library/all/2016/02/neuroendocrine-pancreatic-tumor-treatment-options
    If you’re able to attain an 80% reduction in metastatic volume of the liver, essentially a metastasectomy of the liver, then you are doing a quite significant benefit to the patient’s long-term survival. […] Another modality that can be used is interventional radiology and radio frequency ablation, essentially just burning the areas of the liver that would have remnant tumor in certain areas. […] Neuroendocrine tumors that have metastasized to the liver can be treated in a variety of ways. In addition to those that I’ve mentioned, surgical resection and burning them, liver transplantation is also an option in selective cases.
  • #10 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/non-functioning/treatment
    NETs grow at different rates, but they often grow very slowly. So you might not need treatment straight away. […] Surgery is the main treatment for non functioning pancreatic NETs. But surgery is not always possible. Some NETs might have already started to spread. Or you may not be well enough to have an operation. […] A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT). […] The treatment you have depends on: where the NET is and its size, how many tumours you have, whether it has spread to other parts of the body, your general health and fitness, the symptoms you have. […] Your doctor will discuss your treatment. And tell you about its benefits and the possible side effects. […] Your NET might grow very slowly. And it might not grow at all for months or years.
  • #10 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/non-functioning/treatment
    Your doctor might tell you that you don’t need treatment straight away. Your team keeps an eye on your NET with regular scans. They call this watch and wait or surveillance. […] Surgery to remove the NET is usually the main treatment for non functioning NETs of the pancreas. Your surgeon might suggest you have surgery even if they can’t completely remove the cancer. Taking away as much of the cancer as possible can control the NET for some time and help with symptoms. […] Some of these are major operations and there are risks. There are guidelines that help doctors decide who should have surgery. If your NET is smaller than 2cm and isn’t causing other problems, your doctor might not recommend surgery. Talk to your doctor about the risks and benefits of your surgery. […] You might have other treatments if: you cant have surgery to remove the NET, the NET has spread to another part of the body, the NET came back after the initial treatment.
  • #11 Pancreatic Neuroendocrine Tumors (NETs) Pancreatic Cancer | Symptoms, Diagnosis & Treatment | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/pancreatic-cancer/pancreatic-neuroendocrine-tumors.html
    Some pancreatic NETs grow so slowly that the risks of treating may outweigh the risks of not treating for a period of time. In these cases, careful observation can be used as a treatment strategy. […] When pancreatic NETs are contained within the pancreas, the best course of treatment is surgery. […] MD Anderson doctors have played major roles in the approval of several pancreatic NET treatment options, including everolimus, octreotide, lanreotide, and Lu 177 dotatate.
  • #11 Pancreatic Neuroendocrine Tumors (NETs) Pancreatic Cancer | Symptoms, Diagnosis & Treatment | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/pancreatic-cancer/pancreatic-neuroendocrine-tumors.html
    Several types of treatments are used for pancreatic NETs, including: […] The best way to treat pancreatic NETs that are contained within the pancreas is through surgery. Many cases can be cured this way. […] Hormone therapy can be used when surgery is not possible. This therapy uses somatostatin analogues, such as octreotide or lanreotide, to slow a tumors growth. […] Chemotherapy can be used to stop a tumor from growing, and to shrink larger tumors. […] Targeted therapies are a type of precision medicine. They slow or stop the growth of cancer by targeting specific features on cancer cells. […] Peptide receptor radionuclide therapy (PRRT) was recently approved to treat somatostatin-receptor positive pancreatic NETs. […] When pancreatic NETs spread to the liver, liver-directed therapy can be used.
  • #12 8 Innovative Neuroendocrine Tumor Treatment Options | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/neuroendocrine-tumors/neuroendocrine-tumor-treatment.html
    If all of the patients tumors cannot be removed through surgery, a complete cure is not possible. Instead, the disease is managed through treatments designed to slow or stop its progression. […] The treatments and treatment plans for a NET depend on many factors. These include the location of the tumor, whether it is functional or non-functional, and how advanced the disease is. […] Since NETs can be slow growing, doctors may recommend active surveillance. With this approach, the patient undergoes regular tests, including blood, urine and imaging exams, to see if the tumor is growing. No treatments are given unless and until the disease progresses. This is a popular approach for older patients who might find surgery or other therapies difficult to bear. […] Many neuroendocrine tumors can be removed with surgery, especially if they are diagnosed before they have metastasized, or spread beyond the main tumor and nearby lymph nodes. If the surgeon cannot remove the entire tumor, only part of it may be removed in order to help treat a patients tumor-related symptoms.
  • #12 8 Innovative Neuroendocrine Tumor Treatment Options | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/neuroendocrine-tumors/neuroendocrine-tumor-treatment.html
    The patients surgical options will depend on the tumors location. […] Traditional chemotherapy works by killing cells that multiply quickly, whether normal or cancerous. Targeted therapy, also called precision medicine, is different. It works by stopping or slowing the growth or spread of cancer. […] Radiation therapy uses focused beams of energy to destroy cancer cells. […] Peptide radionuclide therapy is a method of delivering cancer-killing radiation to a tumor. […] Somostatin analogs are drugs that slow tumor growth and help relieve cancer symptoms. […] Liver directed therapies use a catheter sent through an artery to deliver treatments directly to the metastasis. […] When neuroendocrine tumors occur in the gastrointestinal tract, they most commonly spread to the liver. Although the surgery becomes more complex, it can still be an option.
  • #13 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/non-functioning
    NETs grow at different rates, but they often grow very slowly. So you might not need treatment straight away. […] Surgery is the main treatment for non functioning pancreatic NETs. But surgery is not always possible. Some NETs might have already started to spread. Or you may not be well enough to have an operation. […] A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT). […] The treatment you have depends on: where the NET is and its size, how many tumours you have, whether it has spread to other parts of the body, your general health and fitness, the symptoms you have. […] Your doctor will discuss your treatment. And tell you about its benefits and the possible side effects. […] Your NET might grow very slowly. And it might not grow at all for months or years.
  • #13 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/non-functioning
    Your doctor might tell you that you don’t need treatment straight away. Your team keeps an eye on your NET with regular scans. They call this watch and wait or surveillance. […] Surgery to remove the NET is usually the main treatment for non functioning NETs of the pancreas. Your surgeon might suggest you have surgery even if they can’t completely remove the cancer. Taking away as much of the cancer as possible can control the NET for some time and help with symptoms. […] Some of these are major operations and there are risks. There are guidelines that help doctors decide who should have surgery. If your NET is smaller than 2cm and isn’t causing other problems, your doctor might not recommend surgery. Talk to your doctor about the risks and benefits of your surgery. […] You might have other treatments if: you can’t have surgery to remove the NET, the NET has spread to another part of the body, the NET came back after the initial treatment.
  • #13 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/non-functioning
    These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options: somatostatin analogues – drugs include octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) – this uses a radioactive medicine to treat the NET, chemotherapy drugs include temozolomide, fluorouracil, capecitabine, streptozotocin, targeted cancer drugs drugs include everolimus and sunitinib. […] For cancer that has spread to your liver you might have: radiofrequency ablation and microwave ablation, surgery to remove cancer from your liver, a liver transplant this is a rare treatment for NETs. […] Doctors are always trying to improve treatments and reduce the side effects. As part of your treatment, your doctor might ask you to take part in a clinical trial. This might be to test a new treatment or to look at different combinations of existing treatments.
  • #14 Evolving Treatments for Pancreatic Neuroendocrine Cancer (PNETs)
    https://www.mountelizabeth.com.sg/health-plus/article/evolving-treatments-for-pancreatic-neuroendocrine-tumours
    Promising new drugs and therapy have emerged to treat pancreatic neuroendocrine tumours (PNETs). […] From cumulative experience worldwide, we know that extensive surgery can prolong the survival of patients, provided it can be performed safely. However, surgery is risky and also comes with a high risk of tumour recurrence. […] Hormonal therapy using a somatostatin analogue mimics the body’s natural hormone somatostatin and blocks its activity. The treatment ameliorates the symptoms caused by excessive hormones released from the tumour cells, as well as control the growth of PNETs. […] Studies have shown that the use of a somastostatin analogue called lanreotide (Somatuline) in the treatment of advanced PNETs can delay tumour growth and reduced the chance of the tumour growing again by half, when compared with a placebo treatment.
  • #15 Advancements in medical treatment for pancreatic neuroendocrine tumors: A beacon of hope
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11008371/
    Somatostatin analogs (SSAs) provide a valuable palliative option by reducing hormonal secretion as well as the tumor burden. […] Notably, molecular targeted therapies, peptide receptor radionuclide therapy (PRRT), and chemotherapy are typically reserved for patients refractory to SSAs. […] According to recent European Society of Medical Oncology guidelines, the mammalian target of rapamycin (mTOR) inhibitor everolimus is recommended for the treatment of G1/G2 pan-NETs. […] Everolimus in combination with SSAs in advanced and metastatic pan-NETs demonstrated greater benefit than everolimus monotherapy in the RADIANT-1 and RADIANT-3 trials. […] PRRT has emerged as a beacon of hope for patients with metastatic but low-grade pan-NETs where curative surgery is not possible. […] In gastrinomas, where surgical intervention might pose significant risks, medical therapy with high-dose proton pump inhibitor therapy (PPI) with or without SSAs can effectively control symptoms and tumor growth.
  • #15 Advancements in medical treatment for pancreatic neuroendocrine tumors: A beacon of hope
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11008371/
    Insulinomas present a unique challenge, as patients develop hypoglycemia. Diazoxide (50-600 mg daily) is the primary medical therapy, and in refractory cases, glucocorticoids, verapamil, and diphenylhydantoin may be considered. […] For glucagonomas, SSAs are considered first-line treatments, as they have shown remarkable efficacy in controlling hormonal symptoms. […] VIPoma can cause severe and life-threatening diarrhea. Supportive care with intravenous fluids and electrolytes is crucial. SSAs have shown substantial promise in improving VIPoma-associated symptoms. […] Management of crisis due to carcinoid syndrome is a medical emergency that requires the infusion of octreotide along with serotonin antagonists such as ondansetron, cyproheptadine, and ranitidine. […] Elucidating the genetic underpinnings of different pan-NET subtypes opens new avenues for tailored therapies. […] The landscape of pan-NET treatment has evolved significantly, offering patients a more tailored and effective approach.
  • #16 Drug therapy for neuroendocrine tumours (NETs) | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/treatment/drug-therapy
    Drug therapy is commonly used to treat and manage neuroendocrine tumours (NETs). Drugs are given for different reasons. You may have drug therapy to: […] slow down and control the growth of the cancer […] destroy neuroendocrine cancer cells […] relieve or control the symptoms of neuroendocrine cancer (palliative therapy). […] Somatostatin analogues are drugs that lower the amount of hormones made and released by NETs. They are mainly used to control symptoms of carcinoid syndrome. […] Somatostatin analogues are also used to slow down and control the growth of advanced or metastatic cancer when surgery cant be done. […] Octreotide LAR or lanreotide is standard treatment for the long-term control of symptoms and tumour growth. The drug is given once a month. It is usually taken for as long as it works. It may be taken for the rest of your life.
  • #16 Drug therapy for neuroendocrine tumours (NETs) | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/treatment/drug-therapy
    Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. It is usually only used to treat poorly differentiated neuroendocrine carcinomas when other treatments dont work or cant be used. […] Targeted therapy may be used to treat advanced well-differentiated pancreatic NETs (pNETs) when surgery cant be done and the cancer continues to grow and spread (progressive). The targeted therapy drugs used are sunitinib (Sutent) and everolimus (Afinitor). […] Supportive drugs may be used to manage or prevent problems caused by NETs. Some examples of supportive drugs that may be used include: […] Tell your healthcare team if you have any of the side effects listed above or others you think might be from drug therapy.
  • #17 Advancements in medical treatment for pancreatic neuroendocrine tumors: A beacon of hope
    https://www.wjgnet.com/1007-9327/full/v30/i12/1670.htm
    Advancements in medical treatment for pancreatic neuroendocrine tumors (pan-NETs) emphasize tailored approaches for specific subtypes. Cytoreductive surgery and somatostatin analogs (SSAs) play pivotal roles in managing tumors, while palliative options such as molecular targeted therapy, peptide receptor radionuclide therapy, and chemotherapy are reserved for SSA-refractory patients. Understanding the genetic basis of pan-NETs and exploring immunotherapies could lead to promising avenues for future research. This review underscores the evolving landscape of pan-NET treatment, offering renewed hope and improved outcomes for patients facing this complex disease. […] Somatostatin analogs (SSAs) provide a valuable palliative option by reducing hormonal secretion as well as the tumor burden. The European Neuroendocrine Tumor Society, North American Neuroendocrine Society, and National Comprehensive Cancer Network suggest the initiation of SSA in patients with unresectable, asymptomatic, well-differentiated pan-NETs and a high tumor burden, as SSAs have been shown to improve survival in the PROMID and CLARINET trials.
  • #17 Advancements in medical treatment for pancreatic neuroendocrine tumors: A beacon of hope
    https://www.wjgnet.com/1007-9327/full/v30/i12/1670.htm
    Notably, molecular targeted therapies, peptide receptor radionuclide therapy (PRRT), and chemotherapy are typically reserved for patients refractory to SSAs. According to recent European Society of Medical Oncology guidelines, the mammalian target of rapamycin (mTOR) inhibitor everolimus is recommended for the treatment of G1/G2 pan-NETs. […] PRRT has emerged as a beacon of hope for patients with metastatic but low-grade pan-NETs where curative surgery is not possible. This targeted therapy utilizes radionuclides to deliver systemic treatment. […] In gastrinomas, where surgical intervention might pose significant risks, medical therapy with high-dose proton pump inhibitor therapy (PPI) with or without SSAs can effectively control symptoms and tumor growth. […] Insulinomas present a unique challenge, as patients develop hypoglycemia. Diazoxide (50-600 mg daily) is the primary medical therapy, and in refractory cases, glucocorticoids, verapamil, and diphenylhydantoin may be considered.
  • #18 Advancements in medical treatment for pancreatic neuroendocrine tumors: A beacon of hope
    https://www.wjgnet.com/1007-9327/full/v30/i12/1670.htm
    For glucagonomas, SSAs are considered first-line treatments, as they have shown remarkable efficacy in controlling hormonal symptoms. […] VIPoma can cause severe and life-threatening diarrhea. Supportive care with intravenous fluids and electrolytes is crucial. SSAs have shown substantial promise in improving VIPoma-associated symptoms. […] Management of crisis due to carcinoid syndrome is a medical emergency that requires the infusion of octreotide along with serotonin antagonists such as ondansetron, cyproheptadine, and ranitidine. […] The landscape of pan-NET treatment has evolved significantly, offering patients a more tailored and effective approach. From surgical interventions to targeted therapies and immunomodulatory agents, advancements in medical treatment for pan-NETs represent a beacon of hope for those facing this challenging disease.
  • #19 Targeted Drug Therapy for PNETs | American Cancer Society | American Cancer Society
    https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/treating/targeted-therapy.html
    Targeted drugs work differently from standard chemotherapy (chemo) drugs. These drugs target specific parts of cancer cells. […] The targeted drugs used to treat pancreatic neuroendocrine tumors (NETs) by blocking angiogenesis (the growth of new blood vessels that nourish cancers) or other important proteins in cancer cells that help them grow. […] Cabozantinib blocks several tyrosine kinases (cell function regulators such as RET, MET, and VEGFR2) and can help slow tumor growth in different ways. This drug is a pill taken once a day. […] Sunitinib blocks several tyrosine kinases and attacks new blood vessel growth. It has been shown to help slow tumor growth. This drug is a pill taken once a day. […] Everolimus blocks a protein known as mTOR, which normally helps cells grow and divide. Everolimus has been shown to help treat advanced pNETs. Everolimus is a pill taken once a day.
  • #19 Targeted Drug Therapy for PNETs | American Cancer Society | American Cancer Society
    https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/treating/targeted-therapy.html
    Belzutifan is an HIF inhibitor. It blocks a protein called hypoxia-inducible factor 2 alpha (HIF-2a), which affects how a cell responds to low oxygen levels (hypoxia) and how new blood vessels form in tumors. This drug is a pill, taken typically once a day. […] Belzutifan can be used in people with von Hippel-Lindau (VHL) disease who have an advanced pNET. […] To learn more about how targeted drugs are used to treat cancer, see Targeted Cancer Therapy.
  • #20 Pancreatic Neuroendocrine Tumors Overview: Comprehensive 2025 Insights – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/pancreatic-neuroendocrine-tumors
    Targeted therapy is a major advancement in the treatment of pancreatic neuroendocrine tumors (PNETs), offering a more focused approach than traditional chemotherapy. While chemotherapy affects both healthy and cancerous cells, targeted therapies are designed to interfere with specific molecules or pathways that cancer cells use to grow, divide, and spread. This more refined strategy helps slow tumor progression while generally causing fewer side effects. […] Everolimus (Afinitor): The efficacy of everolimus in treating advanced pancreatic neuroendocrine tumors was established in the RADIANT-3 trial. This Phase III study demonstrated that everolimus significantly improved progression-free survival (PFS) compared to placebo (Yao JC, et al. Everolimus for advanced pancreatic neuroendocrine tumors.New England Journal of Medicine. 2011).
  • #21 Pancreatic Neuroendocrine Tumors Overview: Comprehensive 2025 Insights – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/pancreatic-neuroendocrine-tumors
    Sunitinib (Sutent): A pivotal Phase III trial confirmed that sunitinib also significantly extended PFS in patients with advanced PNETs. The study reported a median PFS of 11.4 months for sunitinib versus 5.5 months for placebo (Raymond E, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors.New England Journal of Medicine. 2011). […] Peptide Receptor Radionuclide Therapy (PRRT) has become a vital treatment for patients with advanced pancreatic neuroendocrine tumors (PNETs) that express somatostatin receptors. PRRT works by using radiolabeled peptidesmost notably Lutetium-177 dotatate (Lu-177)that bind to somatostatin receptors on tumor cells and deliver targeted radiation to kill cancer cells, minimizing damage to surrounding healthy tissues. […] This approach is often described as a heat-seeking missile because the radiolabeled drug specifically targets tumor cells, sparing most normal tissues. It has been particularly useful for patients with well-differentiated, unresectable, or metastatic NETs that are no longer responding to standard therapies.
  • #21 Pancreatic Neuroendocrine Tumors Overview: Comprehensive 2025 Insights – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/pancreatic-neuroendocrine-tumors
    Researchers are now exploring combination strategies to improve outcomes. This includes using immunotherapy alongside other treatments like peptide receptor radionuclide therapy (PRRT) or targeted therapies. These combinations may enhance the immune systems ability to detect and destroy tumor cells. For example, damage caused by PRRT may expose more tumor antigens, making immunotherapy more effective. […] The therapeutic landscape for pancreatic neuroendocrine tumors (PNETs) has recently expanded with new treatment approvals and promising clinical trials, offering renewed hope for patients with this challenging disease. […] The Phase III CABINET trial demonstrated that cabozantinib significantly improves progression-free survival in patients with advanced pancreatic neuroendocrine tumors (pNETs). Patients receiving cabozantinib had a median PFS of 13.8 months vs. 4.4 months with placebo (HR, 0.23; NEJM, 2024). These results led to its FDA approval in March 2025 for previously treated, advanced well-differentiated pNETs.
  • #21 Pancreatic Neuroendocrine Tumors Overview: Comprehensive 2025 Insights – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/pancreatic-neuroendocrine-tumors
    Immunotherapy is a groundbreaking type of cancer treatment that uses the bodys own immune system to recognize and destroy cancer cells. It has transformed the treatment landscape for several cancers like melanoma, lung, and kidney cancer. However, in pancreatic neuroendocrine tumors (PNETs), the role of immunotherapy is still evolving and remains under active investigation. […] Unlike some aggressive cancers that respond well to immune-based treatments, PNETs are generally slow-growing and dont often trigger strong immune responses. This makes them less responsive to standard immunotherapies like immune checkpoint inhibitors. These drugssuch as nivolumab (anti-PD-1) and pembrolizumabwork by blocking proteins that cancer cells use to hide from the immune system. In early studies, checkpoint inhibitors have shown limited success in PNETs, particularly in well-differentiated tumors. However, some high-grade or poorly differentiated neuroendocrine carcinomas may benefit more, especially if they express high levels of PD-L1 or have a high tumor mutational burden.
  • #22 FDA Approves New Treatment for PNETs: What You Need to Know  – Pancreatic Cancer Action Network
    https://pancan.org/news/fda-approves-new-treatment-for-pnets-what-you-need-to-know/
    The U.S. Food & Drug Administration has granted approval for a new drug to treat pancreatic neuroendocrine tumors, also called PNET or PanNET. […] “I am delighted that patients with pancreatic neuroendocrine tumors now have another treatment option,” said PanCAN Chief Scientific and Medical Officer Anna Berkenblit, MD, MMSc. […] CABOMETYX is a targeted therapy. It has been granted approval by the FDA for a specific subset of patients with pancreatic cancer who are diagnosed with a PNET. […] CABOMETYX is approved for adults with a PNET who have received previous treatment, who are ineligible for surgery and who have “well-differentiated pancreatic neuroendocrine tumors.” […] A Phase III clinical trial tested the benefit of CABOMETYX. […] The overall response rate, or the percentage of PNET patients in the study whose tumors responded to CABOMETYX, was 18%.
  • #22 FDA Approves New Treatment for PNETs: What You Need to Know  – Pancreatic Cancer Action Network
    https://pancan.org/news/fda-approves-new-treatment-for-pnets-what-you-need-to-know/
    People with a PNET diagnosis should talk to their healthcare team about this treatment option. […] In addition to targeted therapy, other options include radiation therapy, liver directed therapy, hormone therapy, chemotherapy and clinical trials. They may be used alone or together. […] This is the first new treatment approved for PNETs since 2018.
  • #23 Shedding light on pancreatic neuroendocrine tumors – UChicago Medicine
    https://www.uchicagomedicine.org/forefront/cancer-articles/shedding-light-on-pancreatic-neuroendocrine-tumors
    The NETs care team at University of Chicago Medicine includes experts across many specialties. They work together to accurately diagnose neuroendocrine tumors, and to determine the most effective treatment for each patient. […] At UChicago Medicine, treatment for NETs often includes surgery, even for tumors that have spread to other organs. Our surgeons are some of the most experienced in the country at treating NETs, and to use unique state of the art techniques to destroy and remove tumors. […] In addition, we were one of the first centers in the country to offer a promising treatment called Peptide Receptor Radionuclide Therapy, or PRRT. PRRT combines a targeting molecule that binds to specific receptors on tumor cells and a radioactive particle that destroys cancer cells. PRRT is given to patients through an IV infusion, and has fewer side effects than other chemotherapies. A course of treatment typically includes four doses given eight weeks apart.
  • #23 Shedding light on pancreatic neuroendocrine tumors – UChicago Medicine
    https://www.uchicagomedicine.org/forefront/cancer-articles/shedding-light-on-pancreatic-neuroendocrine-tumors
    The most common treatment is surgery. If the tumor is still localized and has not spread, we remove tumors greater than 2 centimeters. […] If the tumor has spread, surgery is still common. Here at UChicago Medicine, we are very good at and specialized in this type of surgery. […] This is called parenchymal-sparing resection, which allows us to take out parts of the tumor carefully, one-by-one. The deeper tumors can be burned with microwave ablation. […] Unlike adenocarcinoma, classic chemotherapy is rarely used to treat pNETs. In addition to or instead of surgery, some patients received intramuscular injections or a daily oral pill to slow down the tumor growth, which sometimes is successful for many years. Recent, more advanced therapies include the new and promising PRRT therapy, or peptide receptor radionuclide therapy. PRRT is a radioactive infusion given every other month for eight months that targets the tumor cells without having to affect other organs. It has minimal side effects and is a promising therapy because it can shrink the tumor in up to 30-40% of patients with pancreatic neuroendocrine tumors. […] We also specialize in trying combination therapies, such as combining PRRT with surgery. Also, next year, we will be starting a clinical trial specifically examining these combination therapies for patients with pancreatic neuroendocrine tumors.
  • #24 Neuroendocrine Tumor Treatment – UChicago Medicine
    https://www.uchicagomedicine.org/cancer/types-treatments/neuroendocrine-tumors/treatment
    During surgery, doctors use a probe that emits microwaves to heat and destroy the tumor’s cells. […] If a tumor has reached the critical mass of almost completely replacing the liver, a liver transplant may be an option. […] When other treatments may not be suitable, cancer cell-killing drugs may be recommended. […] The newest advances in targeted therapy for NETs are available at UChicago Medicine. […] PRRT is an innovative theranostics treatment in which synthetic proteins capable of targeting specific cells are combined with radioactive material. […] This creates radiopeptides, which are then injected into a patient’s bloodstream to deliver targeted radiation to NET cells. […] UChicago was one of the first centers in the nation to offer this therapy and has since developed a very robust and experienced PRRT treatment program.
  • #25 Pancreatic Neuroendocrine Tumors Overview: Comprehensive 2025 Insights – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/pancreatic-neuroendocrine-tumors
    Clinical evidence supporting PRRTs effectiveness was established in the landmark NETTER-1 trial, a multicenter, randomized phase III study. The trial demonstrated that patients with midgut NETs treated with Lu-177 dotatate plus octreotide had a progression-free survival (PFS) of 28.4 months, compared to 8.5 months in the control group receiving high-dose octreotide alone (Strosberg J, et al. N Engl J Med. 2017). Although the trial primarily focused on midgut NETs, its results have guided clinical use in other somatostatin receptor-positive tumors, including pancreatic NETs. […] In clinical practice, PRRT is most suitable for patients whose tumors demonstrate strong uptake on Ga-68 DOTATATE PET/CT scans. This ensures that the therapy will specifically target cancer cells expressing somatostatin receptors. PRRT has been shown to not only prolong disease control but also improve quality of life in many cases (National Cancer Institute, 2024).
  • #25 Pancreatic Neuroendocrine Tumors Overview: Comprehensive 2025 Insights – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/pancreatic-neuroendocrine-tumors
    A randomized Phase II trial demonstrated that the combination of capecitabine and temozolomide (CAPTEM) was significantly more effective than temozolomide alone in treating advanced pancreatic neuroendocrine tumors. The study found a median progression-free survival (PFS) of 22.7 months for CAPTEM compared to 14.4 months for temozolomide monotherapy. […] Liver metastases are common in PNETs and are often managed with interventional radiologic therapies. Salem et al. (2012) demonstrated that Yttrium-90 (90Y) radioembolization for hepatic metastases of neuroendocrine tumors provided effective tumor control and prolonged survival in many patients (Salem R, et al. Cancer. 2012). […] In more advanced or refractory cases, liver transplantation may be considered. A European multicenter study by Mazzaferro et al. (2016) found that while liver transplantation was a viable option, patients with PNETs had lower 5-year survival compared to those with other primary tumor sites (Mazzaferro V, et al. Ann Surg. 2016).
  • #25 Pancreatic Neuroendocrine Tumors Overview: Comprehensive 2025 Insights – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/pancreatic-neuroendocrine-tumors
    Peptide receptor radionuclide therapy (PRRT) has emerged as a targeted treatment for metastatic, well-differentiated PNETs. Lutetium Lu 177 dotatate (Lutathera) delivers targeted radiation to tumor cells expressing somatostatin receptors. The NETTER-1 trial demonstrated a 54.4% increase in estimated PFS at 20 months for patients treated with Lutathera in combination with somatostatin analogs compared to somatostatin analogs alone. […] Research into novel therapies for PNETs is ongoing. A clinical trial is investigating the combination of surufatinib, a targeted therapy, with tislelizumab, an immunotherapy agent, for neuroendocrine tumors in the lungs, pancreas, and gastrointestinal tract. This approach aims to enhance the immune systems ability to recognize and attack tumor cells. […] Early-phase trials are exploring personalized mRNA vaccines designed to elicit an immune response against specific tumor antigens in pancreatic cancer patients. Preliminary results have shown that some patients remained cancer-free for extended periods post-treatment. […] Given that approximately 90% of pancreatic cancers harbor KRAS mutations, targeted therapies like daraxonrasib are under investigation. Early trials have shown that patients with stage-four pancreatic cancer experienced significant responses, with extended survival times.
  • #26 Treatment Patterns of Pancreatic Neuroendocrine Tumor (pNET) Patients at Two Canadian Cancer Centres
    https://www.mdpi.com/1718-7729/32/2/86
    In 2022, the Canadian Agency for Drugs and Technologies in Health (CADTH) officially recommended funding for lutetium-based PRRT therapy for unresectable or metastatic pNETs. […] This highlights the importance of equitable access to this important treatment modality so that patients can receive the treatment that is most in keeping with the current literature on pNETs.
  • #27 Pancreatic cancer – Wikipedia
    https://en.wikipedia.org/wiki/Pancreatic_cancer
    If the tumor is not amenable to surgical removal and is causing symptoms, targeted therapy with everolimus or sunitinib can reduce symptoms and slow progression of the disease. […] Standard cytotoxic chemotherapy is generally not very effective for PanNETs, but may be used when other drug treatments fail to prevent the disease from progressing, or in poorly differentiated PanNET cancers. […] Radiotherapy is occasionally used if there is pain due to anatomic extension, such as metastasis to bone. […] Palliative care focuses not on treating the underlying cancer, but on treating symptoms such as pain or nausea, and can assist in decision-making, including when or if hospice care will be beneficial.
  • #28 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment – NCI
    https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq
    Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. […] Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. […] Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery. […] Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. […] Supportive care is given to lessen the problems caused by the disease or its treatment. […] Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: Surgery to remove the tumor, Chemotherapy, Hormone therapy, Targeted therapy, Regional chemotherapy, Hepatic arterial occlusion or chemoembolization, with or without systemic chemotherapy.
  • #29 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente
    https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.pancreatic-neuroendocrine-tumors-islet-cell-tumors-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062794
    If technically and medically feasible, primary management of endocrine tumors of the pancreas involves surgical resection with curative intent. […] Surgery plays a role even in the setting of metastatic disease. The symptoms of metastatic functional pancreatic neuroendocrine tumors (NETs) may be ameliorated by the reduction of overall tumor burden through surgical debulking. […] Somatostatin analogues may be effective in reducing the symptoms of functional tumors. […] Chemotherapy using drugs such as the following, either alone or in combination, has been shown to have antitumor effects, but evidence is weak or conflicting regarding the impact of chemotherapy on overall survival: Streptozocin, Doxorubicin, Fluorouracil, Chlorozotocin, Dacarbazine, Temozolomide. […] A variety of systemic agents have shown biological or palliative activity, including: Tyrosine kinase inhibitors (e.g., sunitinib), Temozolomide, Vascular endothelial growth factor pathway inhibitors, Mammalian target of rapamycin inhibitors (e.g., everolimus).
  • #30 Pancreatic Neuroendocrine Tumour Treatments
    https://neuroendocrine.org.au/treatments/treatment-for-pancreatic-neuroendocrine-tumours/
    Somatostatin analogues, such as octreotide and lanreotide, are effective in inhibiting hormone secretion and tumour growth. […] Everolimus and sunitinib are targeted therapies that inhibit specific signalling pathways involved in tumour growth and angiogenesis. […] PRRT is an innovative treatment option for advanced PNETs, particularly those expressing somatostatin receptors. […] Chemotherapy is indicated for high-grade PNETs / NECs and cases resistant to conventional therapies. […] TACE and SIRT are specialised interventions targeting liver metastases in PNET patients. […] Radiotherapy plays a minimal role in the treatment of Pancreatic Neuroendocrine Tumours (PNETs), particularly in cases where surgery is not possible or when residual tumour remains after surgery. […] Combining different treatment modalities, such as surgery, chemotherapy, targeted therapy, and radiation, forms the cornerstone of multimodal approaches to PNET treatment.
  • #31 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq
    If technically and medically feasible, primary management of endocrine tumors of the pancreas involves surgical resection with curative intent. […] Surgery plays a role even in the setting of metastatic disease. The symptoms of metastatic functional pancreatic neuroendocrine tumors (NETs) may be ameliorated by the reduction of overall tumor burden through surgical debulking. […] Somatostatin analogues may be effective in reducing the symptoms of functional tumors. […] Chemotherapy using drugs such as the following, either alone or in combination, has been shown to have antitumor effects, but evidence is weak or conflicting regarding the impact of chemotherapy on overall survival. […] Nearly all of the evidence of activity is derived from case series. […] The approach to treatment often depends on the results of preoperative localization studies and findings at exploratory laparotomy.
  • #32 Pancreatic Neuroendocrine Tumors: A Lesser Threat than Adenocarcinomas, but Still Hard to Treat – Cancer Commons
    https://cancercommons.org/latest-insights/pancreatic-neuroendocrine-tumors-a-lesser-threat-than-adenocarcinomas-but-still-hard-to-treat/
    At this time, biological therapies for PNETs are limited to the drugs sunitinib and everolimus, both FDA-approved for treatment of advanced PNETs based on the results of phase III clinical trials. […] Treating PNETs with a combination of chemotherapy drugs usually has a palliative goal; that is, it may prolong survival, but does not usually cure PNETs. Chemotherapy is recommended for control of bulky or rapidly progressing disease. Streptozocin is a chemotherapy drug that is FDA-approved for PNETs, but it is associated with high toxicity.
  • #33 Pancreatic neuroendocrine tumors // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/pancreatic-neuroendocrine-tumors
    Slowing blood flow to the liver. Slowing blood flow through the liver’s main artery, called the hepatic artery, can slow or stop cancer growth. Other blood vessels in the liver provide enough blood for the rest of the liver to keep working. Often, chemotherapy medicines or radioactive beads are used to block the artery. These methods slow the blood flow and deliver treatment directly to the cancer cells in the liver. […] Destroying cancer cells with heat. Radiofrequency ablation is a procedure that uses energy waves to heat up cancer cells and cause them to die. It’s done using a special probe with tiny electrodes that’s inserted through the skin and into the liver. Energy waves pass through the probe and cause the tissue around it to heat up.
  • #34 Pancreatic Neuroendocrine Tumor | Cedars-Sinai
    https://www.cedars-sinai.org/health-library/diseases-and-conditions/p/pancreatic-neuroendocrine-tumor.html
    Chemotherapy […] Hormone therapy […] Targeted therapy […] Radiofrequency ablation, or using heat to destroy the tumor […] Cryosurgical ablation, or freezing the cancer cells. […] Your healthcare provider uses medicines to help manage your symptoms from the pancreatic NET. These may include: […] Medicines for stomach ulcers […] Therapy or over-the-counter remedies to deal with diarrhea […] IV fluids to help prevent dehydration […] Medicines or other strategies to keep your blood sugar in a healthy range. […] Surgery is the main treatment.
  • #34 Pancreatic Neuroendocrine Tumor | Cedars-Sinai
    https://www.cedars-sinai.org/health-library/diseases-and-conditions/p/pancreatic-neuroendocrine-tumor.html
    Your treatment choices depend on the type of pancreatic NET you have, test results, if the tumor is cancer, and the stage of the cancer. The goal of treatment may be to cure you, control the tumor or cancer, or to help ease problems caused by the tumor or cancer. Talk with your healthcare team about your treatment choices, the goals of treatment, and what the risks and side effects may be. […] Surgery is the most common treatment, if the tumor can be removed. The type of surgery depends on how big the tumor is and where it is in the pancreas. Nearby tissues, such as parts of the pancreas, stomach, small intestine, or liver may also need to be removed. […] For tumors that cant be removed by surgery, or that have spread to other parts of the body, other treatments may be used to shrink the tumor, destroy it, or control the symptoms its causing. These include:
  • #35 Pancreatic Neuroendocrine Tumors (Islet Cell) | Memorial Sloan Kettering Cancer Center
    https://www.mskcc.org/cancer-care/types/gastrointestinal-neuroendocrine/pancreatic-neuroendocrine-islet-cell-tumors
    Chemotherapy is usually reserved for patients whose pancreatic neuroendocrine tumors begin to grow during treatment with octreotide, or if a patient has symptoms from the tumor that are not well controlled by octreotide. In addition, chemotherapy may be used to treat tumors that contain fast-growing (undifferentiated) cells. External-beam radiation therapy, which delivers radiation from a machine outside of the body, is typically only used to treat patients with pancreatic neuroendocrine tumors that are causing symptoms such as pain, particularly when they have metastasized to the bone. […] Some pancreatic neuroendocrine tumors absorb a hormone called norepinephrine. These tumors may respond to a nuclear medicine technique called MIBG radiolabeled therapy. In this treatment, the patient is given an intravenous dose of MIBG, a protein that is similar to norepinephrine and is attached to a radioactive substance. The MIBG is absorbed by the tumor, permitting the radioactive substance to selectively destroy tumor cells.
  • #35 Pancreatic Neuroendocrine Tumors (Islet Cell) | Memorial Sloan Kettering Cancer Center
    https://www.mskcc.org/cancer-care/types/gastrointestinal-neuroendocrine/pancreatic-neuroendocrine-islet-cell-tumors
    Researchers at Memorial Sloan Kettering are exploring new treatment approaches using a mouse model that exhibits pancreatic neuroendocrine tumors. This model will be used to conduct early-stage testing of new drug therapies and targeted antibody-based treatments. The pancreatic neuroendocrine tumor model also will be used to test a new class of drugs that may have the potential to block the production of a protein called cathepsin proteases, which are thought to promote the growth of pancreatic neuroendocrine tumors.
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  • #37 Pancreas – Neuroendocrine Cancer UK
    https://www.neuroendocrinecancer.org.uk/neuroendocrine-cancer/neuroendocrine-cancer-by-primary-secondary-sites/pancreas/
    Welcome to our dedicated webpage focusing on pancreatic neuroendocrine cancer. Here, we aim to provide you with a comprehensive understanding of this complex disease. Delve into our comprehensive guide, for information about different types of pancreatic neuroendocrine neoplasms, insights into symptoms, diagnostic tests, treatment, and grading. Follow-up, and factors contributing to the development of pancreatic neuroendocrine cancer. […] Pancreatic Enzyme Replacement Therapy (PERT) is the primary treatment and significantly improves survival and quality of life (QoL) in PEI patients. […] Pancreatic enzyme replacement therapy (PERT) is the cornerstone of treatment and is associated with improved survival and quality of life (QoL) in patients with PEI.
  • #38 Neuroendocrine Tumors – Pancreatic Cancer  |  Johns Hopkins Pathology
    https://pathology.jhu.edu/pancreas/neuroendocrine
    The multidisciplinary team at Johns Hopkins is dedicated to providing the best cutting-edge care to our patients with neuroendocrine tumors of the pancreas. […] Neuroendocrine tumors are treated very differently from other cancers in the body. […] Neuroendocrine tumors are treated in very specific ways. Because some neuroendocrine tumors grow slowly, many neuroendocrine tumors are treated surgically. Other therapies specifically target unique receptors made by neuroendocrine tumors. […] In an exciting advance, the FDA today approved Lutetium for the treatment of neuroendocrine tumors, including neuroendocrine tumors of the pancreas. Lutetium is a peptide receptor radionucleotide therapy. It targets somatostatin receptor positive neuroendocrine tumors. The approval is based on a randomized phase 3 clinical trial.
  • #39 Pancreatic Neuroendocrine Tumors – Pancreas Disease | UCLA Health
    https://www.uclahealth.org/medical-services/pancreas-disease/pancreatic-conditions/pancreatic-neuroendocrine-tumors
    UCLA offers some of the most advanced imaging and diagnosis available for pancreatic neuroendocrine tumors, including: […] While a pancreatic cancer diagnosis can be scary, patients should know there are many 5-year survivors, even for those with advanced stages of the disease. New treatments and drugs offer a better chance for long-term survival, and there is hope for a cure. […] Pancreatic cancer treatment typically involves some combination of surgery, chemotherapy and/or radiation: […] Radioligand therapy (Peptide receptor radionucleotide therapy PRRT): This is a state-of-the-art therapy that is targeted specifically to tumor cells. Radioactive nucleotides are linked to peptides and antibodies that bind to tumor cells and cause their destruction. UCLA is one of the only centers in the country to offer this treatment, including clinical trials for patients with all stages of pancreatic neuroendocrine tumors.
  • #40 Pancreatic Neuroendocrine Tumors | Center for Advanced Digestive Care – NewYork-Presbyterian/Weill Cornell Medical Center
    https://www.nyp.org/cadc/neuroendocrine-tumors/pancreatic-neuroendocrine-tumors
    Immunotherapy is designed to boost the body’s natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to bolster, target, or restore the ability of the immune system to recognize and destroy cancer cells. […] This treatment targets faulty genes or proteins that contribute to cancer growth and development. Early results of clinical trials with targeted drugs for the treatment of neuroendocrine tumors are promising, but additional research is needed to confirm their effectiveness.
  • #41 Exploring Immunotherapy for Carcinoid and Pancreatic Neuroendocrine Tumors | Rutgers Cancer Institute of New Jersey
    https://www.cinj.org/exploring-immunotherapy-carcinoid-and-pancreatic-neuroendocrine-tumors
    A clinical trial testing the immunotherapy drug pembrolizumab is showing the drug to be well tolerated among patients who have carcinoid or pancreatic neuroendocrine tumors. […] With treatment options being limited for patients with carcinoid and pancreatic neuroendocrine tumors, it is imperative to explore new therapy options for these populations. […] At the time our results were reported we discovered findings similar to other trials of immunotherapy agents, with the majority of patients actually not responding to therapy. 12 percent of patients with carcinoid tumors and six percent of patients with pancreatic neuroendocrine tumors experienced a response to therapy. However, patients who achieved response were likely to have durable control of their disease, with all responses greater than or equal to six months in duration. Therapy was overall well tolerated and safe, with some side effects related to autoimmune processes caused by the medication. […] These findings are interesting but need further validation in larger studies of patients with carcinoid and pancreatic neuroendocrine tumors.
  • #42 Pancreatic neuroendocrine tumors – Care at Mayo Clinic – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pancreatic-neuroendocrine-tumors/care-at-mayo-clinic/mac-20352492
    Mayo Clinic offers a truly integrated practice, with a variety of specialists regularly working together and meeting to discuss cases. This ensures that you receive comprehensive care that takes into account all of your needs. Your care team carefully weighs all of your treatment options to create a plan that’s personalized for you. […] The range of treatments offered to people with pancreatic neuroendocrine tumors includes surgery, targeted therapy, chemotherapy, radiofrequency ablation and peptide receptor radionuclide therapy (PRRT). […] People who seek care at Mayo Clinic have access to the latest treatments, including PRRT with lutetium Lu 177 dotatate (Lutathera). Mayo Clinic doctors participated in the clinical trial that led to the approval of this treatment in the United States.
  • #42 Pancreatic neuroendocrine tumors – Care at Mayo Clinic – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pancreatic-neuroendocrine-tumors/care-at-mayo-clinic/mac-20352492
    What I think Mayo has to offer is really being a full service station, if you will, for all things pancreatic. Where there’s really a team approach to diagnosing and treating neuroendocrine tumors. […] So changes in the treatment of pancreatic neuron tumors have definitely occurred over the last several years. For the early stage, PNET patients who have small tumors, low-grade tumors as seen on the biopsy, we can sometimes wait and we don’t always have to operate. And then most recently we have the PRRT, or the peptide receptor radionuclide therapy, probably the most promising treatment out there. So this treatment results in tumor shrinkage in probably as high as 20% to 30% of the patients. The treatment is well tolerated. It’s very safe. […] Patients with neuron tumors who are seen at large volume specialized centers seem to do better than the average patients out there. So I think the treatment at Mayo may result in a better outcome.
  • #43
    https://link.springer.com/article/10.1007/s13193-024-01908-y
    Pancreatic neuroendocrine tumors (PanNETs) account for approximately 2% of all pancreatic malignancies. Several systemic treatment options have been developed over the last four decades, ranging from cytotoxic chemotherapy and octreotide to newer targeted therapies like sunitinib, cabozantinib, and lenvatinib. […] Although surgery or liver-directed therapy remains cornerstone for management of metastatic PanNETs, however, they remain unfeasible in majority of cases. The optimal sequence of the systemic therapies for the advanced PanNETs depends mainly on the tumor burden, Ki-67 index, and the tempo of the disease. […] In the end, drawing from ENETS (European Neuroendocrine Tumor Society) and ESMO (European Society for Medical Oncology) guidelines, we propose a working algorithm for the management of advanced PanNETs, not amenable to surgery or liver-directed therapies.
  • #44 Innovating treatment options for pancreatic neuroendocrine cancer
    https://www.bswhealth.com/blog/innovating-treatment-options-for-pancreatic-neuroendocrine-cancer
    One exciting recent advancement comes in the fight against pancreatic neuroendocrine cancer. […] A breakthrough radiopharmaceutical treatment, or liquid radiation, recently approved by the Food and Drug Administration (FDA), is bringing new hope. […] One exciting recent advancement is lutetium Lu 177 dotatate, a type of liquid radiation that works by targeting a high dose of radiation at the cancerous cells in the pancreas. […] In clinical trials, patients who received this treatment saw a 79 percent reduction in the progression of their disease, meaning this treatment has the potential to significantly slow the growth of pancreatic neuroendocrine tumors. […] Approved by the FDA in January 2018, this treatment is a game-changer and a more targeted alternative to chemotherapy and radiation. […] But it is an extremely useful tool for oncologists to add to the tool belt to slow down the progression of this disease. […] In the meantime, we will continue to push forward promising new treatments and therapies to help give people the best possible odds in fighting this disease.
  • #45 The Difference Between Pancreatic Cancer and Pancreatic Neuroendocrine Tumors | Henry Ford Health – Detroit, MI
    https://www.henryford.com/blog/2022/11/pancreatic-cancer
    Sometimes, patients can live with neuroendocrine tumors for a long time, which is why Dr. Philip says it’s important to utilize the help of a multidisciplinary team so that you’re surrounded by people who are familiar with this rare disease to not only treat it, but also to help maintain your quality of life in the process.